Rad III MT1 - Synovial Sarcoma, Childhood Leukemia, Hypertrophic Pulmonary Osteoarthropathy, osteochondroma, HME

age for synovial sarcoma

15-40 yo

what is synovial sarcoma

malignancy of soft tissue

syonvial sarcoma most commonly seen where

posterior compartment of knee

hip

shoulder/ankle

does synovial sarcoma communicate with the joint space?

NO

what does synovial sarcoma mimic

monoarticular arthritis

what will xray show with synovial sarcoma

slowly enlargening nodular soft tissue mass

possible calcification near a joint

physical findings for synovial sarcoma

large palpable mass- encapsulated synovial fluid

what is given first consideration when you come across a large soft tissue mass in gluteal or knee region - not coming from bone and you see that it has calcification within it

synovial sarcoma

why do you see the ST mass of synovial sarcoma

because the cellularity is dense

treatment for synovial sarcoma?

amputation is likely

% of cases where bone is involved with synovial sarcoma?

30%

what is the MC malignant childhood disease?

leukemia

where does childhood leukemia target in the body

bone marrow - disease of WBC

what is the MC form of childhood leukemia

Acute lymphoblastic lymphoma (ALL)

% of cases that ALL accounts for

80%

Age for childhood leukemia

2-5 yo

Classic symptoms for childhood leukemia

joint pn, fever, weakness, lethargy, pallor, loss of appetite, chronically sick

labs and physical findings

elevated ESR

lymphadenopathy

splenomegaly

hepatomegaly

radiographic features of childhood leukemia

generalized diffuse osteoporosis

metaphyseal bands in long bones

bilaterally symmetric just adjacent to growth plate

periostitis

lystic destruction

growth arrest lines

hypertrophic (pulmonary) osteoarthropathy (HOA) causes where on the bone to react?

diaphyseal periosteal thickening

where is HOA MC found

lower extremity

classic triad associated with HOA (hypertrophic pulmonary osteoarthropathy)

1) digita clubbing

2) symmetric arthritis

3)metaphyseal/diaphyseal periostitis occurring as a sequelae to a major visceral disorder usually intrathoracic

MC cause of HOA

bronchogenic carcinoma

aka for GCT

osteoclastoma

why is GCT considerd quasimalignant

80% benign

20% malignant

what location has a higher incidence of malignant GCT

distal radius

why does GCT get so big so quickly

extremely vascularized, lyse through bone and expand - osteoclastic in nature

what does a lack of sclerotic border out well-defined lytic lesions lead us to think?

Giant Cell Tumor

GCT in females

more commonly benign

GCT in males

malignancy shows in higher instances

age for GCT

20-40

description of GCT

expansile, pain producing, soap bubble lesion, eccentric, in metaphysis and expanding to epiphysis

does GCT expand to the epiphysis?

yes

MC sites of involvement for GCT

distal femur, proximal tib, distal radius, proximal humerus

MC benign neoplasm of patella and sacrum

giant cell tumor

does GCT usually disrupt the cortex?

no - it thins it but does not disrupt generally when benign

can you differentiate between benign and malignant GCT from xray?

no

why do you need to do CT for GCT

to be sure cortex is still there

name sign for GCT on bone scan?

Doughnut sign- increased uptake around periphery

there is a higher risk of what with GCT due to higher vascularization?

secondary ABC formation

name sign associated with ABC

fluid fluid level sign

two shapes of osteochondroma

peduculated and sessile

S/S of osteochondroma

usually asymptomatic unless they disturb surrounding blood vessels or nerves

MC complaint about osteochondroma

painless hard mass/bump/lump near a joint

pain and growth of osteochondroma signals what?

malignant degneration

MC sites of involvement for osteochondroma

femur, humerus, tibia, ribs, scapula

osteochondroma on the scapula is called

Luschka's tubercle

what is the MC primary benign bone tumor of the appendicular skeleton

osteochondroma

what is an osteochondroma

bony exostosis on external surface of bone with a hyaline cartilaginous cap

age for osteochondroma?

before 20

gender associated with osteochondroma

male

osteochondroma is believed to be

beginning with an outgrowth from epiphyseal cartilage

where is peduculated osteochondroma usually found

distal femur

where on the bone are osteochondromas found

meta-diaphyseal

sessile shaped osteochondromas are commonly found

humerus and scapula

what is HME

hereditary multiple exostosis

do you have increased chance for malignancy if you have HME

yes, 20% chance of malignant transformation

coat hanger exostoses are associated with what

osteochondroma

key radiologic feature of osteochondroma

cortex and spongiosa of ostechondroma and host bone blend imperceptibly

a large lobulated peduculated cauliflower mass found in the meta-diaphyseal region of the femur appears to be

osteochondroma

dense spinal osteochondromas may be called

cauliflower spine

how is HME inherited

autosomal dominant

is HME bilateral and symmetric

yes

bayonette hand deformity is associated with

HME

characteristics of bayonette hand deformity

shortened ulna

outward bowing of radius

subluxation of radioulnar jt

what should you suspect if pt starts complainting of pain where there is an osteochondroma

osteosarcoma, fibrosarcoma, chondrosarcoma

specific type of osteochondroma in the nails

subungal exostosis

where does subungal exostosis usually present?

distal portion of terminal phalanx of toes

is the subungal exostosis of metaphyseal origin

no